2410例肝窦阻塞综合征患者临床特征的系统分析

Clinical characteristic of hepatic sinusoidal obstruction syndrome in 2 410 patients:a systematic analysis

目的 分析总结2 410例肝窦阻塞综合征(hepatic sinusoidal obstruction syndrome,HSOS)患者的临床特点、诊断和治疗情况。方法 在中国知网、万方和维普数据库中检索发表于1980年1月至2022年8月的文献,最终纳入150篇文献,共计2 410例HSOS患者,分析总结本组HSOS患者的性别、年龄、病因、临床表现、实验室检查结果、影像学表现、病理学特征、治疗方式和预后。结果 2 410例HSOS患者主要病因与服用含有吡咯烷生物碱(pyrrolidine alkaloids,PAs)的食物、植物及各种药物如土三七有关。临床表现以腹胀(78.09%)、腹水(45.61%)、腹痛(24.78%)、肝肿大(26.14%)、黄疸(30.77%)多见。典型影像学表现为肝脏肿大、不均匀强化且增强后呈“地图状”或斑片状改变、肝静脉显示不清或未显示及下腔静脉受压狭窄。实验室检查主要表现为AST、ALT、GGT及TBIL水平轻至中度升高,ALB和PLT降低。HSOS的治疗方式包括内科保守治疗、使用低分子肝素或华法林抗凝治疗、TIPS分流手术、肝脏移植术等。结论 HSOS好发于男性,病因以服用土三七为主,常有腹胀、腹痛、腹水、肝肿大等突出临床表现,实验室检查和影像学检查可协助诊断,治疗以对症、抗凝、TIPS和肝移植为主,多数患者预后良好。

Objective To analyze and summarize the clinical features,diagnosis and treatment of 2 410 patients with hepatic sinusoidal obstruction syndrome(HSOS).Methods Chinese and foreign literatures published from Jan.1980 to Aug.2022 were retrieved from CNKI,WanFang and VIP databases.A total of 2 410 HSOS patients and 150 published papers were enrolled.The gender,age,etiology,clinical manifestations,laboratory results,imaging findings,pathological features,treatment conditions and prognosis of HSOS patients were analyzed and summarized.ResultsThe main etiology of 2 410 HSOS patients was related to the consumption of plants,foods and various drugs containing pyrrolidine alkaloids(PAs) such as Tusanqi.The most common clinical manifestations were abdominal distension(78.09%),ascites(45.61%),abdominal pain(24.78%),hepatomegaly(26.14%) and jaundice(30.77%).The typical imaging manifestations were hepatomegaly.The liver was not uniformly enhanced and the enhancement was ″map-like″ or patchy.Hepatic vein display was not clear or not,and the inferior vena cava was compressed stenosis.In laboratory examination,AST,ALT,GGT and TBIL levels were mainly to moderately elevated,ALB and PLT decreased.Treatments of HSOS include conservative medical treatment,anticoagulation with low molecular weight heparin or warfarin,TIPS shunt surgery and liver transplantation.Conclusion HSOS tends to males.The etiology of HSOS is mainly the use of Tusanqi,often show abdominal distension,abdominal pain,ascites and hepatomegaly,and other prominent clinical manifestations.Laboratory examination and imaging examination can assist in the diagnosis,the treatment of HSOS is mainly symptomatic,anticoagulation,TIPS and liver transplantation.What′s more,most patients have a good prognosis.